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| {{Infobox_Disease |
| | #redirect [[Creutzfeldt-Jakob disease]] |
| Name = Kuru |
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| Image = |
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| Caption = |
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| DiseasesDB = 31861 |
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| ICD10 = {{ICD10|A|81|8|a|80}} |
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| ICD9 = {{ICD9|046.0}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = 001379 |
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| MeshID = D007729 |
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| }}
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| {{SI}}
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| {{CMG}}
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| {{SK}} Laughing sickness
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| ==Overview==
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| '''Kuru''' is an incurable degenerative [[neurological disorder]] (brain disease) that is a type of [[transmissible spongiform encephalopathy]] found in humans. It causes physiological as well as neurological effects that ultimately lead to death. It was endemic among the Fore tribe of Papua New Guinea and was confined to the Fore population and those nearby populations with whom they intermarried. It is characterized by truncal [[ataxia]], preceded by headaches, joint pains and shaking of the limbs, with progression to death over approximately 18 months for the most susceptible victims. It is believed to be caused by [[prion]]s and is related to [[Creutzfeldt-Jakob disease]].<ref name="Collinge">{{cite journal | author= Collinge J, Whitfield J, McKintosh E, et al | title =Kuru in the 21st century—an acquired human prion disease with very long incubation periods | journal =Lancet | volume =367 | issue =9528 | pages =2068–74 | publisher = | month =June | year =2006 | pmid=16798390 | doi =10.1016/S0140-6736(06)68930-7 }}</ref> It is best known for the [[epidemic]] that occurred in Papua New Guinea in the middle of the twentieth century.<ref>[http://www.ninds.nih.gov/disorders/kuru/kuru.htm Kuru Information Page: National Institute of Neurological Disorders and Stroke (NINDS)<!-- Bot generated title -->]</ref>
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| == Historical Perspective ==
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| The word ''kuru'' is taken from the Fore word "kuria/guria", 'to shake'. It is also known as the ''laughing sickness'' due to the pathologic bursts of laughter people would display when afflicted with the disease.
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| Kuru is a rare and fatal brain disorder that occurred at epidemic levels during the 1950s-60s among the Fore people in the highlands of New Guinea. The disease was the result of the practice of ritualistic cannibalism among the Fore, in which relatives prepared and consumed the tissues (including brain) of deceased family members. <ref>[http://www.as.ua.edu/ant/bindon/ant570/Papers/McGrath/McGrath.htm Kuru: The Dynamics of a Prion Disease<!-- Bot generated title -->]</ref> Brain tissue from individuals with kuru was highly infectious, and the disease was transmitted either through eating or by contact with open sores or wounds. Government discouragement of the practice of cannibalism led to a continuing decline in the disease, which has now mostly disappeared.
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| The disease was researched by [[Daniel Carleton Gajdusek]] who was awarded (along with [[Baruch S. Blumberg]]) the [[Nobel Prize in Physiology or Medicine]] in 1976 for his showing that kuru was transmissible to chimpanzees. This was the first time that this group of encephalopathies had been demonstrated to be infectious and therefore a major step forwards in their investigation. As kuru is the only [[epidemic]] of human [[prion]] disease in known human history, it has provided important insights into the variant CJD.
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| == Pathophysiology ==
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| Kuru belongs to a class of infectious diseases called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. The hallmark of a TSE disease is misshapen protein molecules that clump together and accumulate in brain tissue. Scientists believe that misshapen prion proteins have the ability to change their shape and cause other proteins of the same type to also change shape. Other TSEs include Creutzfeldt-Jakob disease and fatal familial insomnia in humans, bovine spongiform encephalopathy in cattle (also known as mad cow disease), scrapie in sheep and goats, and chronic wasting disease in deer and elk.
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| Kuru is a latent disease which manifests after an incubation period of 5 - 20years. This phase in asymptomatic phase of incubation period. The disease progress in three stages.
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| a. Ambulent stage
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| b. Sedentary stage
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| c. Terminal stage
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| During the progression of disease mobility decreases significantly.
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| ==Epidemiology and Demographics==
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| It was endemic in the Fore tribe of New Guinea.
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| ===Gender===
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| It was 8-9 times more prevalent in women and children than men.
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| ==Diagnosis==
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| ===Symptoms===
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| * [[Headache]]
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| * [[Joint pains]]
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| * Shaking of limbs
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| ===Physical Examiation===
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| ====Neurologic====
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| * [[Ataxia]]
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| * [[Tremors]]
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| * Unsteady [[gait]]
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| * Decreased muscle control
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| * Deterioration of speech
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| * Dysarthria (slurred speech)
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| * Incapable of walking without support
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| * Emotionally unstable
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| * [[Depression]]
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| ====Other===
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| * Uncontrolled sporadic laughter
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| * [[Ulcerations]] (sores with pus and necrosis)
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| ==Treatment==
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| There were no treatments that could control or cure kuru, other than discouraging the practice of cannibalism. Currently, there are no cures or treatments for any of the other TSE diseases.
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| What is the prognosis?
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| Similar to other the TSEs, kuru had a long incubation period; it was years or even decades before an infected person showed symptoms. Because kuru mainly affected the cerebellum, which is responsible for coordination, the usual first symptoms were an unsteady gait, tremors, and slurred speech. (Kuru is the Fore word for shiver.) Unlike most of the other TSEs, dementia was either minimal or absent. Mood changes were often present. Eventually, individuals became unable to stand or eat, and they died in a comatose state from 6 to 12 months after the first appearance of symptoms.
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| == References ==
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| {{reflist|2}}
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| {{Prion diseases}}
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| [[Category:Neurology]]
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| [[Category:Prions]]
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| [[Category:Transmissible spongiform encephalopathies]]
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| [[Category:Disease]]
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| [[bg:Куру (болест)]]
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| [[cs:Kuru]]
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| [[de:Kuru (Krankheit)]]
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| [[es:Kuru]]
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| [[eo:Kuruo]]
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| [[fr:Kuru (maladie)]]
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| [[ko:쿠루병]]
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| [[hr:Kuru]]
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| [[it:Kuru (malattia)]]
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| [[he:מחלת קורו]]
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| [[lt:Kuru (liga)]]
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| [[nl:Kuru (ziekte)]]
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| [[no:Kuru]]
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| [[pl:Kuru]]
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| [[pt:Kuru]]
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| [[ru:Куру (болезнь)]]
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| [[simple:Kuru (disease)]]
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| [[sr:Куру]]
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| [[fi:Kuru (tauti)]]
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| [[sv:Kuru (sjukdom)]]
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